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CASE REPORT
Year : 2018  |  Volume : 7  |  Issue : 1  |  Page : 57-59

Pickering syndrome in a dialysis patient: A case report with review of pathophysiology


Department of Nephrology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India

Date of Web Publication22-Mar-2018

Correspondence Address:
Dr. B Sangeetha Lakshmi
Department of Nephrology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JDRNTRUHS.JDRNTRUHS_105_17

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  Abstract 


Flash pulmonary edema (FPE) is a rare clinical presentation of renal artery stenosis. It is an episode of abrupt onset pulmonary edema that resolves rapidly. It occurs in patients with bilateral renal artery stenosis or unilateral renal artery stenosis with a solitary functioning kidney. We report a patient of diabetic end-stage renal disease on maintenance hemodialysis who manifested FPE on a few occasions which responded to renal revascularization. The indications of renal revascularization have been narrowed to poor blood pressure control, when progressive loss of renal function is encountered, or when circulatory congestion is a prominent feature.

Keywords: Flash pulmonary edema, hemodialysis, Pickering syndrome, renal artery stenosis


How to cite this article:
Lakshmi B S, Nagaraj R D, Chaitanya V, Reddy M H, Kumar AC, Sridhar A V, Kishore C K, Kumar V S. Pickering syndrome in a dialysis patient: A case report with review of pathophysiology. J NTR Univ Health Sci 2018;7:57-9

How to cite this URL:
Lakshmi B S, Nagaraj R D, Chaitanya V, Reddy M H, Kumar AC, Sridhar A V, Kishore C K, Kumar V S. Pickering syndrome in a dialysis patient: A case report with review of pathophysiology. J NTR Univ Health Sci [serial online] 2018 [cited 2020 Mar 29];7:57-9. Available from: http://www.jdrntruhs.org/text.asp?2018/7/1/57/228140




  Introduction Top


Flash pulmonary edema (FPE) is a rare clinical presentation of renal artery stenosis. It is an episode of abrupt onset pulmonary edema that resolves rapidly. It occurs in patients with bilateral renal artery stenosis or unilateral renal artery stenosis with a solitary functioning kidney.[1] It was reported in only 2 patients (1%) out of 148 patients with renal artery stenosis who were treated with stent insertion.[2] Here, we report a patient of diabetic end-stage renal disease on maintenance hemodialysis who manifested FPE on a few occasions, which responded to renal revascularization.


  Case Report Top


A 66-year-old gentleman a patient of diabetic end-stage renal disease with hypertension had been on thrice weekly 4-h bicarbonate in-center hemodialysis. About one and half years since being on hemodialysis, he manifested sudden episodes of pulmonary edema necessitating urgent dialysis in addition to regular scheduled dialysis. He required four classes of antihypertensive drugs to maintain blood pressure at 160/90 mmHg. There were no renal bruits on examination. An echocardiography demonstrated concentric left ventricular hypertrophy, left atrial enlargement, adequate left ventricular systolic function, and diastolic dysfunction (grade 2) with an ejection fraction of 55%. There was also mild mitral and aortic regurgitation. His residual renal function was approximately 0.8 L of urine per day. However, considering the possibility of FPE secondary to cardiovascular and renovascular disease, he was evaluated with coronary and renal angiographies. The coronary angiography revealed insignificant double vessel disease in left anterior descending and right coronary arteries. A computed tomography (CT) angiogram revealed bilateral renal artery stenosis. He was subjected to renal angiogram via the right femoral artery, which showed ostial lesion of the left renal artery occluding it up to 90%. The right renal artery lesion was insignificant. A renal angioplasty and stenting of the left renal artery was performed [Figure 1]. Following the procedure, the patient became free from FPE episodes. The number of classes of antihypertensives reduced to two and urine improved to 1.0 L per day. After multiple avascular accesses failures, the patient is being maintained on peritoneal dialysis in our facility.
Figure 1: Left renal artery stent (arrow). Calcification of aorta and the peritoneal catheter are also visible

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  Discussion Top


Pickering et al. in 1988 described acute recurrent pulmonary edema in association with renal artery stenosis.[3] It differs from the usual causes of left ventricular failure with pulmonary edema in that this condition is usually not associated with severe left ventricular systolic dysfunction. It is mostly nocturnal and occurs rather suddenly.[4] FPE is a general term used to describe a particularly dramatic form of acute decompensated heart failure. Regardless of its etiology, an acute increase in left ventricular end diastolic pressure is the condition sine qua non for the development of FPE, and remains the common denominator of all clinical situations associated with it. However, what distinguishes FPE from other forms of decompensated heart failure is that, because of unique underlying pathophysiological mechanisms, flooding of alveolar space can occur within minutes resulting in an acute life threatening emergency. Bilateral renal artery stenosis predisposes patients to develop FPE by three main pathophysiological mechanisms. (a) defective natriuresis; (b) increased hemodynamic burden and exacerbation of diastolic dysfunction; and (c) failure of the pulmonary capillary blood gas barrier.[5]

There are differences in pathophysiology between unilateral and bilateral renal artery stenosis. The 2-kidney-1-clip hypertension model corresponds to unilateral renal artery stenosis. In unilateral renal artery stenosis, one renal artery decreases ipsilateral renal perfusion thereby activating the renin–angiotensin–aldosterone cascade and causing sodium and fluid retention. As the contralateral kidney is functioning normally, it compensates for the increase in blood pressure by suppressing renin secretion and augmenting sodium excretion; so-called pressure natriuresis ensues. This escape mechanism is imperfect in patients with bilateral renal artery stenosis. The 1-kidney-1-clip hypertension model corresponds to bilateral renal artery stenosis. The pressure natriuresis cannot take place in bilateral renal artery stenosis as the function of both kidneys is impeded and intravascular volume expansion secondary to sodium and water retention persists.[5],[6]

Pulmonary capillary stress failure and increased pulmonary vascular permeability are often unconsidered pathogenic mechanisms of FPE. The rapid and exaggerated increase in left ventricular pressure transmitted to left atrium and pulmonary veins and to the unprotected pulmonary capillaries gives rise to increased pulmonary vascular permeability and pulmonary edema. When the intracapillary pressure exceeds 20–25 mmHg, the edema fluid leaks through the endothelial barrier and floods the interstitial and alveolar space. Angiotensin II, catecholamines, and endothelin-1 may increase the permeability of alveolar capillary barrier.[5]

FPE is usually associated with bilateral renal artery stenosis or stenosis of a single surviving kidney, and very rarely it had been reported, only one report, involving unilateral stenosis with bilateral functioning kidneys.[1] Our patient had disappearance of episodes of FPE after renal angioplasty of left kidney, which requires explanations. The frequent episodes of FPE in our patient even though he had significant disease on one side could have two-fold explanations, (a) The right kidney might not be functioning at all rendering him critically dependent on left kidney for natriuresis. With a significant lesion in left renal artery deprived him of this aid. (b) An initial small increase in the left end-diastolic volume precipitated by activation of the renin–angiotensin–aldosterone system may be associated with a marked elevation in the diastolic pressure of patients with severe diastolic dysfunction. This contributed to the development of pulmonary edema as in this patient.

The combination of hypertension and volume retention occurring because of altered intrarenal hemodynamics and aldosterone-mediated salt and water retention are the major contributors to the development of pulmonary edema with bilateral renal artery stenosis. In patients of end-stage renal disease, all these factors are frequently present and do participate in precipitating pulmonary edema.[7]

It is interesting to note that, in majority of studies, patients experience FPE only at night. It is suggested that these patients might have severe renal artery lesions which are noncritical during the day with normal ambulant blood pressure but with a nocturnal dip in blood pressure, the lesion becomes critical provoking severe perturbation of renin angiotensin system.[8]

To conclude, after the publication of prospective trials,[9],[10],[11] the indications of renal revascularization have narrowed. At present, initiating relatively aggressive antihypertensive therapy including agents that block the renin–angiotensin system and other measures to slow atherosclerosis underpins of treatment of renovascular hypertension. When blood pressure control is poorly achieved, when progressive loss of renal function is encountered, or when circulatory congestion is a prominent feature associated with stenosis to the entire functioning renal mass, many would prefer renal revascularization.[12]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Noh HJ, Jo HC, Yang JH, Kim SM, Lee HJ, Choi JH, et al. Flash pulmonary edema in a patient with unilateral renal artery stenosis and bilateral functioning kidneys. Korean Circ J 2010;40:42-5.  Back to cited text no. 1
[PUBMED]    
2.
Perkovic V, Thomson KR, Mitchell PJ, Gibson RN, Atkinson N, Field PL, et al. Treatment of reno-vascular disease with percutaneous stent insertion: Long-term outcomes. Australas Radiol 2001;45:438-43.  Back to cited text no. 2
[PUBMED]    
3.
Pickering TG, Herman L, Devereux RB, Michael JF, Herman SL, Sotelo JE, et al. Recurrent pulmonary edema in hypertension due to bilateral renal artery stenosis: Treatment by angioplasty or surgical intervention. Lancet 1988;2:551-2.  Back to cited text no. 3
    
4.
Koduganti SC, Kumar R. Bilateral renal artery stenosis presenting as flash pulmonary edema. JAPI 2006;54:651-4.  Back to cited text no. 4
    
5.
Messerli FH, Bangalore S, Makani H, Rimoldi SF, Allemann Y, White CJ, et al. Flash pulmonary oedema and bilateral renal artery stenosis: The Pickering syndrome. Eur Heart J 2011;32:2231-5.  Back to cited text no. 5
[PUBMED]    
6.
Garovic VD, Textor SC. Renovascular Hypertension and ischemic nephropathy. Circulation 2005;112:1362-74.  Back to cited text no. 6
[PUBMED]    
7.
Headley CM, Wall BM. Flash pulmonary edema in patients with chronic kidney disease and end stage renal disease. Nephrology Nurs J 2007;34:15-28.  Back to cited text no. 7
    
8.
Mansoor S, Shah A, Scoble JE. Flash pulmonary oedema – a diagnosis for both cardiologist and the nephrologist?. Nephrol Dial Transplant 2001;16:1311-3.  Back to cited text no. 8
    
9.
Bax L, Woittiez AJ, Kouwenberg HJ, Mali WP, Buskens E, Beek FJ, et al. Stent placement in patients with atherosclerotic renal artery stenosis and impaired renal function: A randomized trial. Ann Intern Med 2009;150:840-8.  Back to cited text no. 9
    
10.
ASTRAL Investigators, Wheatley K, Ives N, Gray R, Kalra PA, Moss JG, et al. Revascularization versus medical therapy for renal-artery stenosis. N Engl J Med 2009;20:1953-62.  Back to cited text no. 10
    
11.
Cooper CJ, Murphy TP, Cutlip DE, Jamerson K, Henrich W, Reid DM, et al. Stenting and medical therapy for atherosclerotic renal-artery stenosis. N Engl J Med 2014;370:13-22.  Back to cited text no. 11
    
12.
Textor SC. Renovascular hypertension: Is there still a role for stent revascularization? Curr Opin Nephrol Hypertens 2013;22:525-30.  Back to cited text no. 12
    


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